Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation
Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.
KeywordsRepaired tetralogy of fallot Apical rotation Basal rotation Twist Untwist rate Biventricular function
The authors would like to acknowledge Zhanna Roytman for data collection and Matthew’s Hearts of Hope Research Grant for financial support that was used towards statistical and Tomtec Software support.
Compliance with Ethical Standards
Conflict of interest
Mr. Klas is a full time employee of TomTec Imaging Systems. No disclosures for other authors.
- 1.Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, Gatzoulis MA, Groenink M, Inuzuka R, Kilner PJ, Koyak Z, Landzberg MJ, Mulder B, Powell AJ, Wald R, Geva T (2014) Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of fallot enrolled in the INDICATOR cohort. Heart 100:247–253CrossRefPubMedGoogle Scholar
- 4.Babu-Narayan SV, Diller GP, Gheta RR, Bastin AJ, Karonis T, Li W, Pennell DJ, Uemura H, Sethia B, Gatzoulis MA, Shore DF (2014) Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of fallot and potential prognostic value of preoperative cardiopulmonary exercise testing. Circulation 129:18–27CrossRefPubMedGoogle Scholar
- 6.Friedberg MK, Fernandes FP, Roche SL, Grosse-Wortmann L, Manlhiot C, Fackoury C, Slorach C, McCrindle BW, Mertens L, Kantor PF (2012) Impaired right and left ventricular diastolic myocardial mechanics and filling in asymptomatic children and adolescents after repair of tetralogy of fallot. Eur Heart J Cardiovasc Imaging 13:905–913CrossRefPubMedGoogle Scholar
- 11.Toumanidis ST, Kaladaridou A, Bramos D, Skaltsiotes E, Agrios JN, Vasiladiotis N, Pamboucas C, Kottis G, Moulopoulos SD (2013) Apical rotation as an early indicator of left ventricular systolic dysfunction in acute anterior myocardial infarction: experimental study. Hell J Cardiol 54:264–272Google Scholar
- 16.Notomi Y, Srinath G, Shiota T, Martin-Miklovic MG, Beachler L, Howell K, Oryszak SJ, Deserranno DG, Freed AD, Greenberg NL, Younoszai A, Thomas JD (2006) Maturational and adaptive modulation of left ventricular torsional biomechanics: Doppler tissue imaging observation from infancy to adulthood. Circulation 113:2534–2541CrossRefPubMedGoogle Scholar
- 17.Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 23:465–495 (quiz 576–7)CrossRefPubMedGoogle Scholar
- 18.Valente AM, Cook S, Festa P, Ko HH, Krishnamurthy R, Taylor AM, Warnes CA, Kreutzer J, Geva T (2014) Multimodality imaging guidelines for patients with repaired tetralogy of fallot: a report from the AmericanSsociety of Echocardiography: developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr 27:111–141CrossRefPubMedGoogle Scholar
- 19.Rudski LG, Lai WW, Afilalo J, Hua LQ, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the american society of echocardiography endorsed by the european association of echocardiography, a registered branch of the european society of cardiology, and the canadian society of echocardiography. J Am Soc Echocardiogr 23:685–713CrossRefPubMedGoogle Scholar
- 22.Menting ME, Eindhoven JA, van den Bosch AE, Cuypers JAAE., Ruys TPE, van Dalen BM, McGhie JS, Witsenburg M, Helbing WA, Geleijnse ML, Roos-Hesselink JW (2014) Abnormal left ventricular rotation and twist in adult patients with corrected tetralogy of fallot. Eur Heart J Cardiovasc Imaging 15:566–574CrossRefPubMedGoogle Scholar
- 25.Fernandes FP, Manlhiot C, Roche SL, Grosse-Wortmann L, Slorach C, McCrindle BW, Mertens L, Kantor PF, Friedberg MK (2012) Impaired left ventricular myocardial mechanics and their relation to pulmonary regurgitation, right ventricular enlargement and exercise capacity in asymptomatic children after repair of tetralogy of fallot. J Am Soc Echocardiogr 25:494–503CrossRefPubMedGoogle Scholar
- 26.Weidemann F, Eyskens B, Mertens L, Dommke C, Kowalski M, Simmons L, Claus P, Bijnens B, Gewillig M, Hatle L, Sutherland GR (2002) Quantification of regional right and left ventricular function by ultrasonic strain rate and strain indexes after surgical repair of tetralogy of fallot. Am J Cardiol 90:133–138CrossRefPubMedGoogle Scholar